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伊朗南部依赖输血的地中海贫血患者内分泌紊乱的患病率及其相关因素:一项历史性队列研究。

Prevalence of endocrine disorders and their associated factors in transfusion-dependent thalassemia patients: a historical cohort study in Southern Iran.

机构信息

Nemazee Hospital, Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, 71937-1135, Iran.

Shiraz Endocrinology and Metabolism Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

出版信息

J Endocrinol Invest. 2019 Dec;42(12):1467-1476. doi: 10.1007/s40618-019-01072-z. Epub 2019 Jun 21.

Abstract

PURPOSE

Transfusion-dependent beta-thalassemia (TDT) patients suffer from various endocrinopathies. The main contributing factor associated with these complications is iron overload, secondary to frequent blood transfusions. To improve patients' quality of life, we evaluated the prevalence of endocrine disorders while considering the associated factors for further assessment.

METHODS

Seven hundred thirteen transfusion-dependent thalassemia patients with age range 10-62 years were enrolled in this study. Serum calcium, phosphorous, fast blood sugar, ferritin, 25-OH vitamin D, free thyroxin, thyroid-stimulating hormone and parathyroid hormone were assessed. Bone mineral density was measured by dual-energy X-ray absorptiometry.

RESULTS

In total, 86.8% of the TDT patients suffered from at least one endocrinopathy. The prevalence of endocrinopathies in descending order of frequency was low bone mass (72.6%), hypogonadism (44.5%), diabetes mellitus (15.9%), hypoparathyroidism (13.2%), and hypothyroidism (10.7%). Age, body mass index and splenectomy were significantly associated with most of the endocrine disorders.

CONCLUSION

Endocrine complications are frequently observed in TDT patients. Splenectomy is a major risk factor and should be generally avoided unless it is highly indicated. Periodic surveillance of endocrine function and proper management of iron overload are advised.

摘要

目的

依赖输血的β-地中海贫血(TDT)患者患有各种内分泌疾病。这些并发症的主要相关因素是铁过载,这是由于频繁输血引起的。为了提高患者的生活质量,我们评估了内分泌紊乱的患病率,并考虑了相关因素以进行进一步评估。

方法

本研究纳入了 713 名年龄在 10-62 岁之间的依赖输血的地中海贫血患者。评估了血清钙、磷、快速血糖、铁蛋白、25-羟维生素 D、游离甲状腺素、促甲状腺激素和甲状旁腺激素。通过双能 X 线吸收法测量骨密度。

结果

共有 86.8%的 TDT 患者至少患有一种内分泌疾病。内分泌疾病的患病率按频率降序排列依次为:低骨量(72.6%)、性腺功能减退(44.5%)、糖尿病(15.9%)、甲状旁腺功能减退(13.2%)和甲状腺功能减退(10.7%)。年龄、体重指数和脾切除术与大多数内分泌疾病显著相关。

结论

内分泌并发症在 TDT 患者中经常观察到。脾切除术是一个主要的危险因素,除非高度指征,否则一般应避免。建议定期监测内分泌功能并适当管理铁过载。

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